Spinal intradural malignant peripheral nerve sheath tumor in a child with neurofibromatosis type 2: the first reported case and literature review.

Abstract

Spinal intradural malignant peripheral nerve sheath tumors (MPNSTs) in children are extremely rare, with only five reported cases in the literature. A 9-year-old female with neurofibromatosis type 2 (NF-2) presented with right hip pain and severe weakness of bilateral legs for 3 months. Magnetic resonance (MR) imaging revealed multiple intradural masses at the T11-L2, L4, and L5-S5 level respectively, and bilateral vestibular schwannomas in the cerebellopontine angle. Partial tumor excision with T11-L2 laminectomy was undertaken and the tumors in the spinal cord were consistent with the diagnosis of epithelioid MPNSTs. No adjuvant therapy was performed after surgery. No metastasis of the tumor was found in the 6-month follow-up MR imaging. She died of brain metastasis at 9 months after surgery. MPNSTs should be added to the differential diagnosis of intradural tumors of the pediatric spine, even in children with NF-2. Multidisciplinary treatment consisting of total surgical removal and adjuvant radiotherapy should be considered due to poor prognosis of this abnormality. To our knowledge, this is the first case of spinal MPNSTs in a child with NF-2 to be published in English literature.